Successful staged anatomic repair for congenitally corrected transposition with both atrioventricular valves'...

A 4-year-old boy with dextrocardia, congenitally corrected transposition of the great arteries, pulmonary valve stenosis, and ventricular septal defect demonstrated progressive hypoxemia and mild-to-moderate mitral and tricuspid regurgitation. Cardiac magnetic resonance imaging revealed insufficient right ventricular volume for Senning-Rastelli type anatomical repair. Given the elevated surgical risk associated with mitral regurgitation and inadequate ventricular volume, an initial palliative approach was undertaken at 2 years of age, consisting of systemic-to-pulmonary shunt placement and mitral valve repair, which improved oxygenation and promoted right ventricular growth, with trivial mitral regurgitation. Subsequent imaging confirmed adequate ventricular development and trivial mitral regurgitation, allowing for definitive anatomic repair at 4 years and 7 months of age. The procedure comprised a Senning atrial switch combined with a Rastelli operation. The postoperative course was uneventful, and imaging demonstrated preserved biventricular function with minimal atrioventricular valve regurgitation and no evidence of outflow or pathway obstruction.